Studies in Meconium in Cystic Fibrosis: the Activities of alpha-<i>D</i>-Mannosidase, beta-Glucuronidase, beta-<i>D</i>-Fucosidase Acid and Alkaline Phosphatase

Abstract

The specific activities of α-D-mannosidase, β-glucuronidase, β-D-fucosidase, acid and alkaline phosphatases were studied in meconium from infants with cystic fibrosis (CF) and control subjects. The study revealed significant variations in the specific activity of the enzymes except for acid phosphatase. The variations were not uniform. The activities of α-D-mannosidase, β-glucuron-idase and alkaline phosphatase were markedly decreased (p < 0.001, p < 0.002, p < 0.001, respectively), while the activity of β-D-fucosidase was significantly increased (p < 0.001) in meconium from the infants with CF. It is suggested that the decreased activity of α-D-mannosidase and β-glucuronidase might contribute to the accumulation of the abnormal substances in CF meconium. The highly increased activity of β-D-fucosidase raises the possibility of an additional or alternative method for screening newborns for CF using meconium as the test material.